DOI: 10.1590/1516-3180.2016.0095220616 CASE REPORT Boerhaave syndrome – case report Síndrome de Boerhaave – relato de caso Biljana Radovanovic DinicI, Goran IlicII, Snezana Tesic RajkovicIII, Tatjana Jevtovic StoimenovIV Medical School, University of Niš, and Gastroenterology and Hepatology Clinic, Niš Clinical Center, Niš, Serbia

IMD. Associate Professor and Attending ABSTRACT Physician, Medical School, University of Niš, and CONTEXT: Boerhaave syndrome consists of spontaneous longitudinal transmural rupture of the esopha- Gastroenterology and Hepatology Clinic, Niš gus, usually in its distal part. It generally develops during or after persistent vomiting as a consequence of a Clinical Center, Niš, Serbia. sudden increase in intraluminal pressure in the esophagus. It is extremely rare in clinical practice. In 50% of IIMD. Associate Professor, Medical School, the cases, it is manifested by Mackler’s triad: vomiting, lower thoracic pain and subcutaneous emphysema. University of Niš, and Institute of Forensic Hematemesis is an uncommon yet challenging presentation of Boerhaave’s syndrome. Compared with rup- Medicine, Niš, Serbia. tures of other parts of the digestive tract, spontaneous rupture is characterized by a higher mortality rate. IIIMD. Attending Physician, Gastroenterology and CASE REPORT: This paper presents a 64-year-old female patient whose vomit was black four days be- Hepatology Clinic, Niš Clinical Center, Niš, Serbia. fore examination and became bloody on the day of the examination. Her symptoms included epigastric IVMD. Associate Professor, Medical School, pain and suffocation. Physical examination showed hypotension, tachycardia, dyspnea and a swollen and University of Niš, and Institute of Biochemistry, painful abdomen. Auscultation showed lateral crackling sounds on inspiration. Ultrasound examination Niš, Serbia. showed a distended stomach filled with fluid. Over 1000 ml of fresh was extracted by means of na- sogastric suction. Esophagogastroduodenoscopy was discontinued immediately upon entering the proxi- KEY WORDS: Esophagus. mal esophagus, where a large amount of fresh blood was observed. The patient was sent for emergency Rupture, spontaneous. abdominal surgery, during which she died. An autopsy established a diagnosis of Boerhaave syndrome Hematemesis. and ulceration in the duodenal bulb. . CONCLUSION: Boerhaave syndrome should be considered in all cases with a combination of gastroin- Emphysema. testinal symptoms (especially epigastric pain and vomiting) and pulmonary signs and symptoms (es- pecially suffocation). PALAVRAS-CHAVE: Esôfago. RESUMO Ruptura espontânea. CONTEXTO: A síndrome de Boerhaave é uma ruptura longitudinal transmural espontânea do esôfago, Hematêmese. Pneumotórax. normalmente da parte distal. Ela geralmente se desenvolve durante ou após vômitos persistentes como Enfisema consequência do aumento repentino da pressão intraluminal no esôfago. É extremamente rara na prática clínica. Em 50% dos casos, manifesta-se pela tríade de Mackler: vômitos, dor torácica inferior, enfisema subcutâneo. Hematêmese é uma apresentação incomum porém desafiadora da síndrome de Boerhaave. Em comparação com rupturas de outras partes do tubo digestivo, a ruptura espontânea é caracterizada pela taxa de mortalidade mais elevada. RELATO DO CASO: O artigo apresenta uma paciente do sexo feminino de 64 anos de idade, cujo vômito era preto, quatro dias antes do exame, e continha sangue no dia do exame. Os sintomas incluíam dor epigástrica e sufocação. No exame físico, foi verificada hipotensão, taquicardia, dispneia e abdômen in- chado e doloroso. Ausculta revelou estertores laterais na inspiração. A ultrassonografia mostrou estômago dilatado, preenchido com conteúdo líquido. Sucção nasogástrica evacuou mais de 1.000 ml de sangue fresco. Esofagogastroduodenoscopia foi abortada imediatamente ao se entrar no esôfago proximal, onde foi observada grande quantidade de sangue fresco. A paciente foi encaminhada com urgência para ci- rurgia abdominal, durante a qual faleceu. Autópsia estabeleceu diagnóstico de síndrome de Boerhaave e úlcera no bulbo-duodenal. CONCLUSÃO: A síndrome Boerhaave deve ser considerada em todos os casos com uma combinação de sintomas gastrointestinais (especialmente dor epigástrica e vómitos) e sintomas e sinais pulmonares (especialmente sufocação).

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INTRODUCTION Appropriate therapy was administered (one ampoule of pranto- Boerhaave syndrome consists of spontaneous longitudinal trans- pazole, a total of about 3000 ml of continuous infusion of saline mural rupture of the esophagus. The syndrome is named after solution and lactated Ringer’s solution). The oxygen saturation a German doctor, Herman Boerhaave, who first described it was 95%. A urinary catheter was placed for monitoring diuresis. in 1724.1 In comparison with iatrogenic rupture, which may An electrocardiogram (ECG) showed sinus tachycardia. develop during diagnostic or therapeutic endoscopic procedures, Because of the findings in the abdomen, an ultrasound exam- traumas or various esophageal diseases, spontaneous rupture ination was performed and this showed a distended stomach most commonly develops during or after persistent vomiting, as filled with a large amount of fluid. No free fluid was found in a consequence of a sudden increase in intraluminal esophageal the abdominal cavity. A nasogastric probe was placed in order to pressure. Spontaneous rupture encompasses 15% of all esopha- extract the contents and perform esophagogastroduodenoscopy geal ruptures.2 It is extremely rare in clinical practice. The true (EGD). After inserting the nasogastric probe, about 1,000 ml incidence of Boerhaave syndrome in the general population is of fresh blood was extracted. After the hemodynamic status unknown. However, it is considered to be more common than had improved, esophagogastroduodenoscopy was attempted. once thought, since many cases of Boerhaave syndrome are only Immediately upon insertion of the endoscope into the proximal diagnosed postmortem, thus resulting in underreporting and esophagus, reflux of a large amount of fresh blood was observed; underestimation with regard to both incidence and mortality.1,3 further examination was cancelled. The patient was sent for Boerhaave syndrome is seen most frequently among patients emergency abdominal surgery. However, she died one hour after aged 50-70 years.1 the first examination. The clinical manifestation of spontaneous rupture of the The laboratory findings and coagulation factors, which were esophagus depends on the rupture location. In 50% of the cases, received subsequently, were within normal values. The blood it is manifested by Mackler’s triad: vomiting, lower thoracic pain count showed reduced hemoglobin of 70 g/l (reference values: and subcutaneous emphysema.3,4 115-170 g/l) and increased leukocyte count of 12.0 x 109/l (refer- If the diagnosis is not established in time and if appropriate ence values: 4.0-10.0 x 109/l). therapeutic measures are not undertaken, serious complications The autopsy showed 650 ml of dark red to black thick fluid can develop and this may lead to a poor outcome. Compared content in the right hemithorax and 600 ml in the left hemitho- with ruptures of other parts of the digestive tube, spontaneous rax (Figure 1). The size measurements were 110 x 105 mm. rupture of the esophagus has the highest mortality rate.1,5 The heart weighed 380 g. The thickness of the cardiac muscle of the left ventricle was 18 mm and of the right ventricle, 6 mm. CASE REPORT A rupture along the longitudinal axis was found in the esopha- The patient was a 64-year-old female, with a history of long-term gus, in the posterior left section of the esophageal wall, 15 mm arterial hypertension, who was brought to the Gastroenterology from the cardia. and Hepatology Clinic of the Niš Clinical Center by the emer- The rupture was 30 x 20 mm in size. The esophageal mucosa gency medical services. She was admitted presenting with vom- was smooth and almost completely covered in bloody-black con- iting of fresh blood, black stools, epigastric pain, suffocation and tent (Figure 2). There were no foreign bodies in the abdomi- exhaustion. nal cavity. A small amount of blackish liquid was found in the The problems had first appeared four days before admission in the form of poorly formed black stools and vomiting of small amounts of black substance. She did not see a doctor about these problems. On the day of admission, after vomiting an excessive amount of black substance, she developed a pain in the epigastric region and then began to vomit fresh blood. It was at this stage that she rang the emergency medical services. Physical examination showed that the patient was alert, ady- namic, tachycardiac and easily dyspneic, and her skin was pale. Her blood pressure was 60/40 mmHg. Auscultation of the heart was normal. Auscultation of the lungs showed baseline crackles on inspiration on both sides. The abdomen was tense, especially in the epigastric area and left hypochondrium, with tenderness Figure 1. Macroscopic findings from the intrathoracic contents in the epigastric area. The liver and spleen were of normal size. upon opening the . Note the huge amount of clot.

72 Sao Paulo Med J. 2017; 135(1):71-5 Boerhaave syndrome – case report | CASE REPORT stomach. Numerous small shallow erosions were found in the DISCUSSION fundus and body of the stomach. Spontaneous rupture of the esophagus is a rare clinical entity A mucosal injury of depth 13 mm, covering an area of 20 mm with a high mortality rate.5,6 The pathophysiology of Boerhaave x 15 mm with firm borders and blackish background, consistent syndrome involves a sudden rise in intraluminal esophageal with a duodenal bulb ulcer, was observed (Figure 3). The walls pressure, thereby forcing the gastric contents against a tight cri- were firm and vallum-like and the bottom was partially black. copharyngeus muscle.3,6 It most often develops during or after Greenish and black content was present throughout the intestines. intense vomiting caused by excessive eating or drinking alcohol.7 Chemical and toxicological analysis on samples of organ tis- However, spontaneous rupture of the esophagus may occur in sues, blood and urine did not reveal the presence of any psycho- the absence of predisposing factors. There are cases of spontane- active substances or pesticides. ous esophageal rupture during sleep. In some patients, a muscu- The autopsy report declared that the immediate cause of lar layer was missing and this may point to the possibility of ana- death was hemopneumothorax due to esophageal injury and a tomical predisposition for the development of rupture.1,3 chronic duodenal ulcer. In the literature, there are cases in which the rupture was also associated with gastroesophageal reflux disease (GERD), Barrett’s esophagus, peptic stricture of the esophagus, esophageal dysmotility, paraesophageal hernia or bleeding from a duodenal ulcer, which was the case with our patient.5,8,9 In our patient, the esophageal rupture was a consequence of excessive vomiting due to the bleeding from the duodenal ulcer. Spontaneous rupture may occur just above the diaphragm in the posterolateral wall of the esophagus. Perforations are usually longi- tudinal (0.6-8.9 cm long), with the left side more commonly affected than the right (90%). This is probably due to an anatomical weakness of the left posterolateral aspect of the esophagus just above the dia- phragm. Spontaneous rupture is rare below the diaphragm or in the thoracic part of the esophagus.3,7 In our case, the rupture was located in the distal esophagus, 15 mm from the cardia. The clinical manifestation of Boerhaave syndrome depends on the location of the rupture and the time between its devel- Figure 2. Gross examination of the distal esophagus showing opment and examination. Patients with cervical perforation feel a longitudinal complete rupture 15 mm from the cardia. Note the darkened esophageal mucosa. pain in the and upper half of the thorax. In cases of perfora- tion in the rest of the esophagus, pain is present in the lower part of the thorax and/or upper abdomen. Considering that sponta- neous rupture most often happens in the distal esophagus, the majority of patients have Mackler’s triad of symptoms and signs: vomiting, lower thoracic pain and subcutaneous emphysema.3,4 However, this triad is rare, which may delay the diagnosis.10 In a series of 14 patients with Boerhaave syndrome, only a small per- centage had typical signs and symptoms.3 The symptoms of Boerhaave syndrome can be nonspecific. Compared with Mallory-Weiss syndrome, Boerhaave syndrome is rarely manifested through hematemesis or other signs of gas- trointestinal bleeding, including melena.1,3,6,10,11 In Boerhaave syndrome, the rupture is transmural, which leads to esophageal perforation. In our patient, hematemesis was the chief complaint. To begin with, she was vomiting an excessive amount of black Figure 3. Gross findings from the stomach and duodenum substance as a result of bleeding from ulcers. Excessive vomiting showing deep and wide duodenal ulceration in the led to spontaneous rupture of the esophagus, which manifested duodenal bulb (arrow). as vomiting of fresh blood.

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During physical examination of patients, subcutaneous as Gastrografin, since barium may cause severe . emphysema is observed in 28%-66% within the first 24 hours. Esophagography with Gastrografin is 90% sensitive.7 This finding is significant for the initial diagnosis. More typi- Thoracic computed tomography imaging is indicated for cally, subcutaneous emphysema is found later. Besides typical making the diagnosis in patients who do not tolerate esopha- symptoms, atypical symptoms such as hypotension, tachycar- gography. During the procedure, localized fluid collection is dia, tachypnea, feverishness and cyanosis may also be present.1,7 observed, as well as periesophageal air collection.1,15,16 The role Atypical symptoms may be prevented through timely diag- of EGD in the early diagnostic work-up of patients with sus- nosis. is a significant clinical finding.10 pected esophageal perforation has been disputed.17 EGD is not Pneumomediastinum is suspected when, during lung ausculta- recommended for diagnosing Boerhaave syndrome, since it may tion, crunching sounds that are synchronous with the heartbeat increase the rupture and the amount of air in the are heard (Hamman’s sign). This sign is present in around 20% and pleural space.13 In cases with hematemesis, such as in our of the cases.7 patient, the procedure was attempted in order to ascertain the Esophageal rupture may be followed by serious complica- source of bleeding. tions, of which the most important ones are mediastinitis and The treatment for Boerhaave syndrome is both conservative multiple organ dysfunction. Sepsis may develop within a few and surgical. The goals of pharmacotherapy are to reduce mor- hours. In such cases, the clinical picture is dominated by signs bidity and to prevent complications. Surgical management is and symptoms of sepsis, which additionally prevents timely diag- generally required for both spontaneous rupture and traumatic nosis and appropriate therapeutic measures.6,7,12 perforation.14,18 Endoscopic stent insertion offers a promising Laboratory findings are not specific for diagnosing sponta- alternative. The mortality rate varies depending on the time that neous esophageal rupture. Serum albumin is normal but may has elapsed since development of the rupture and its recognition be low, while the globulin fraction may be normal or slightly and treatment. If treatment is not started within 24 hours from elevated.7 Radiography of the heart and lungs is valuable for the onset of symptoms, the mortality rate is 25%; after 24 hours, the diagnosis. Radiographs usually show signs of pneumome- it is 65%; and after 48 hours, it is 75%-89%.19 diastinum or pneumothorax or hydropneumothorax if pleural We reviewed the literature in Medline, PubMed, Embase and effusion is concurrent.3,13 In cases of perforation of the mid- Lilacs using the English keywords “Esophagus”, “Rupture, sponta- dle third of the esophagus, is present on the neous”, “Hematemesis” and “Pneumothorax”; and the Portuguese right side, while in cases of rupture of the distal esophagus, words “Esôfago”, “Ruptura espontânea”, “Hematêmese” and pleural effusion is present on the left side.5 Diagnostic thora- “Pneumotórax” (Table 1). centesis shows the presence of food remnants, increased amy- lase and pH below 6. The presence of pneumomediastinum CONCLUSION with data including vomiting and chest pain are almost defi- Boerhaave syndrome should be considered in all patients with a nite signs of Boerhaave syndrome. Overall, 10% of chest radio- combination of gastrointestinal symptoms (epigastric pain and graphs are normal.7,14 vomiting) and pulmonary symptoms (suffocation), even when all Esophagography is an important imaging examination the signs and symptoms (lower thoracic pain and subcutaneous for confirming the diagnosis and the location of perforation emphysema) of this disease are absent. Early clinical suspicion because it shows extravasation of contrast into the pleural space. will lead to timely diagnosis and maximize the survival chances The procedure is performed with water-soluble contrast, such for the patient.

Table 1. Literature search in medical databases for case reports on Boerhaave syndrome. The literature search was conducted on May 4, 2016 Database Search strategies Papers found Related papers MEDLINE Esophagus AND Rupture, spontaneous AND Hematemesis 9 2 (via PubMed) AND Pneumothorax AND “case reports” [Publication Type] Embase Esophagus AND Rupture, spontaneous AND Hematemesis 0 0 (via Elsevier) AND Pneumothorax AND “case reports” [Publication Type] (Esofago [DeCs]) OR (esophagus [MeSH]) AND (Ruptura espontanea [DeCs]) OR LILACS (Rupture, spontaneous [MeSH]) AND (Hematemese [DeCs]) OR (Hematemesis 0 0 (via Bireme) [MeSH]) AND (Pneumotorax [DeCs] OR Pneumothorax [MeSH]) AND ” relato de caso”

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